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Clinics in Dermatology 2012Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence... (Review)
Review
Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of complement along the basement membrane zone of perilesional skin. The disorder is accompanied by severe pruritus and polymorphous bullous skin lesions. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence and immunoelectron microscopy tests (linear deposition of C3, with or without immunoglobulin G, along the basement membrane zone, within the lamina lucida, and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin). Enzyme-linked immunosorbent assay for pemphigoid gestationis antibody (BP180) is commercially available. If local treatment fails, systemic corticosteroid therapy should be administered. Oral corticosteroids are the therapeutic mainstay in pregnancy and postpartum. The prognosis is good for mother and child, except that there is a risk of preterm delivery and of moderate fetal growth restriction. Recurrence is possible during subsequent pregnancies. There is no significant maternal morbidity or mortality.
Topics: Adrenal Cortex Hormones; Anti-Bacterial Agents; Autoantigens; Basement Membrane; Dermatologic Agents; Enzyme-Linked Immunosorbent Assay; Female; Humans; Immunoglobulin G; Non-Fibrillar Collagens; Pemphigoid Gestationis; Pregnancy; Pregnancy Outcome; Prenatal Care; Prognosis; Collagen Type XVII
PubMed: 22137226
DOI: 10.1016/j.clindermatol.2011.03.009 -
Medicina Clinica Apr 2020
Topics: Female; Humans; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy; Pregnancy Complications
PubMed: 31506200
DOI: 10.1016/j.medcli.2019.05.037 -
Journal of Gynecology Obstetrics and... May 2022Pemphigoid gestationis (PG), also known as gestational pemphigoid, as it is specifically associated with a pregnancy event, is among the rare pregnancy-related... (Review)
Review
Pemphigoid gestationis (PG), also known as gestational pemphigoid, as it is specifically associated with a pregnancy event, is among the rare pregnancy-related dermatoses, characterised by the formation of autoantibodies against Bullous Pemphigoid antigens 180 and 230 (BP180 and BP230), causing significant damage to the basement membrane of the skin, resulting in marked pruritus and blisters on the abdomen and extremities. Diagnosis of PG is basically made by the characteristic clinical picture and confirmed by immunofluorescence studies and histopathology of a skin biopsy. Treatment, just as for other autoimmune dermatoses, is achieved by corticosteroids with the risk of relapses in subsequent pregnancies. Fetal growth restriction and pre-maturity are potential fetal complications associated with the disease, hence the recommended combined antenatal care by a dermatologist as well as an obstetrician, however, this disease is unlikely to be a source of significant maternal morbidity or mortality.
Topics: Autoantibodies; Female; Humans; Pemphigoid Gestationis; Pregnancy; Pregnancy Complications; Prenatal Care; Pruritus
PubMed: 35385801
DOI: 10.1016/j.jogoh.2022.102370 -
The New England Journal of Medicine Aug 2020
Topics: Adult; Biopsy; Complement C3; Female; Humans; Immunoglobulin Isotypes; Pemphigoid Gestationis; Pregnancy; Skin
PubMed: 32846065
DOI: 10.1056/NEJMicm2000922 -
Seminars in Dermatology Jun 1988
Review
Topics: Adrenal Cortex Hormones; Diagnosis, Differential; Female; Humans; Infant, Low Birth Weight; Infant, Newborn; Pemphigoid Gestationis; Pregnancy; Prognosis
PubMed: 3153431
DOI: No ID Found -
Clinics in Dermatology 2016Pemphigoid gestationis (PG) is the only autoimmune disease exclusively emerging in pregnancy. It belongs to the pemphigoid group of disorders, a class of autoimmune... (Review)
Review
Pemphigoid gestationis (PG) is the only autoimmune disease exclusively emerging in pregnancy. It belongs to the pemphigoid group of disorders, a class of autoimmune blistering skin diseases featuring an immune response against different hemidesmosomal proteins. PG is caused by a break of immunotolerance against the hemidesmosomal protein BP180. Several lines of evidence suggest that this break of immunotolerance is linked to specific maternal major histocompatibility complex (MHC) class II gene variants and aberrant expression of MHC class II molecules in the placenta. The close time association of the emergence of PG with pregnancy and the obviously very short period required from the initial break of immunotolerance to the onset of skin inflammation set PG into a unique position among autoimmune diseases in view of the fact that, for other autoimmune diseases, the time and site of the break of immunotolerance are usually vastly elusive and the period of silent disease can only be speculated on. In this review we highlight the features of PG and summarize current knowledge about its pathogenesis. We believe that this disease offers the best opportunity to elucidate comprehensively all phases of the pathogenesis of an autoantibody-driven disease.
Topics: Female; Humans; Pemphigoid Gestationis; Pregnancy
PubMed: 27265076
DOI: 10.1016/j.clindermatol.2016.02.010 -
Clinics in Dermatology 2006Herpes gestationis, coined by Milton in 1872, or gestational pemphigoid is the most clearly characterized dermatosis of pregnancy. It is a rare vesiculo-bullous eruption... (Review)
Review
Herpes gestationis, coined by Milton in 1872, or gestational pemphigoid is the most clearly characterized dermatosis of pregnancy. It is a rare vesiculo-bullous eruption that develops during the last trimester or even postpartum and creates severe pruritus. Its etiology is unknown, but it is considered as an autoimmune-mediated dermatosis closely related to the pemphigoid group. Herpes gestationis is associated with a positive C3 deposition along the base of the epidermis in salt-split skin, with increased frequency of HLA-DR3 and also the combination DR3 and DR4. It has a high risk of prematurity and disappears in the postpartum period within weeks or months.
Topics: Female; Humans; Pemphigoid Gestationis; Pregnancy
PubMed: 16487884
DOI: 10.1016/j.clindermatol.2005.10.011 -
BMJ Case Reports Feb 2011A 39-year-old woman in the 39th week of her fifth pregnancy presented with severe itching. In recent weeks she had developed an increasingly itchy rash on her trunk and...
A 39-year-old woman in the 39th week of her fifth pregnancy presented with severe itching. In recent weeks she had developed an increasingly itchy rash on her trunk and arms. Upon examination we observed multiple erythematous plaques and vesicles. Histological examinations of two skin biopsies confirmed the diagnosis of pemphigoid gestationis. Because the patient's complaints worsened we decided to deliver the baby, and as it was in transverse lie this was by caesarean section. A healthy son without rash was born. Although pemphigoid gestationis occurs in only 1 in 50,000 pregnancies, it is very important to be aware of this condition when a pregnant woman has itching. Not recognising pemphigoid gestationis may lead to inadequate maternal treatment and possible preterm birth and neonatal pemphigoid gestationis.
Topics: Adult; Female; Humans; Pemphigoid Gestationis; Pregnancy; Pruritus
PubMed: 22715180
DOI: 10.1136/bcr.01.2010.2623 -
Dermatology Online Journal Dec 2013We present a 21-year-old primigravida woman with a several-week history of pruritic, edematous, targetoid plaques that appeared initially on the abdomen, flanks, and...
We present a 21-year-old primigravida woman with a several-week history of pruritic, edematous, targetoid plaques that appeared initially on the abdomen, flanks, and legs and that progressed to involve the inner aspects of the upper arms and lateral aspects of the chest. The histopathologic findings showed perivascular and interstitial dermatitis with eosinophils and vacuolar changes with linear C3 deposition at the basement-membrane zone on direct immunofluorescence study. A diagnosis of pemphigoid gestationis was made. Pemphigoid gestationis is a rare, bullous dermatosis of pregnancy that may be associated with prematurity and small-for-gestational age birth weights. The diagnosis is often made with direct immunofluorescence studies of perilesional skin. Oral glucocorticoids remain the gold standard of therapy in moderate-to-severe cases. The edematous papules and plaques of pemphigoid gestationis may be particularly difficult to distinguish from polymorphic eruption of pregnancy; therefore, immunofluorescence studies are prudent. Prompt recognition and appropriate management may reduce morbidity of this disease, which often recurs with subsequent pregnancies.
Topics: Complement C3; Female; Fluorescent Antibody Technique, Direct; Glucocorticoids; Humans; Pemphigoid Gestationis; Pregnancy; Young Adult
PubMed: 24365006
DOI: No ID Found -
The British Journal of Dermatology Jan 2015
Topics: Female; Humans; Pemphigoid Gestationis; Pregnancy
PubMed: 25581583
DOI: 10.1111/bjd.13457